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Kikuchi-Fujimoto disease following the subacute phase of Kawasaki disease. 川崎病亚急性期后发生菊池-藤本病。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70343
Hiroyuki Nagao, Miki Yoshimura, Daigo Kato, Yo Okizuka
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引用次数: 0
Functional status of pediatric patients after discharge from intensive care units in a middle-income country. 中等收入国家儿童重症监护病房出院后的功能状况
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70300
Regina Melittio Gasparetti, Kelsy Catharina Nema Areco, Heitor Pons Leite, Paulo Cesar Koch Nogueira, Tulio Konstantyner

Background: Patients discharged from the intensive care unit (ICU) often experience high morbidity rates. The aim of the present study was to estimate the incidence of functional capacity impairment in pediatric patients who were discharged from the ICU in a middle-income country and to identify associated factors, considering the socioeconomic context.

Methods: This was a multicenter cohort study of 357 patients aged <18 years admitted to three ICUs in the interior cities of São Paulo State. The Functional Status Scale was used to assess the patients at admission, ICU discharge, and hospital discharge. New morbidity was defined as a change in any domain of the scale equal to or greater than two, or a change in the total score equal to or greater than three. A multiple logistic regression model was used to identify independent associations with new morbidity.

Results: New morbidity occurred in 14.6% and 12.3% of patients at ICU and hospital discharge, respectively, compared with those at admission. The feeding (p < 0.001) and respiratory (p = 0.036) functional domains were the most affected at ICU discharge. The risk factors associated with new morbidity at hospital discharge were heart disease, older age, longer ICU stay, and higher PELOD2 severity score.

Conclusions: To reduce the risk of new functional morbidity, healthcare teams should be attentive to the critically ill pediatric population, particularly those who are older and have chronic clinical conditions, especially heart disease.

背景:从重症监护病房(ICU)出院的患者通常有很高的发病率。本研究的目的是估计中等收入国家从ICU出院的儿科患者功能障碍的发生率,并在考虑社会经济背景的情况下确定相关因素。方法:这是一项包含357例老年患者的多中心队列研究。结果:与入院患者相比,ICU和出院患者的新发病率分别为14.6%和12.3%。结论:为了降低新功能发病的风险,卫生保健团队应关注危重儿科人群,特别是那些年龄较大和有慢性临床疾病,特别是心脏病的儿童。
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引用次数: 0
Correction to "Evaluation of clinical and genetic characteristics of cystic fibrosis patients in the Southeastern Anatolia of Turkey". 更正“土耳其安纳托利亚东南部囊性纤维化患者的临床和遗传特征评估”。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70360
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引用次数: 0
Clinical characteristics of pediatric Moraxella bacteremia in a children's hospital. 某儿童医院小儿莫拉菌血症的临床特点
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70342
Joonho Shin, Meiwa Shibata, Yuho Horikoshi

Background: Moraxella species are commensal organisms of the upper respiratory tract that only occasionally cause invasive infection. Pediatric Moraxella bacteremia is extremely rare, and its clinical features and optimal management remain poorly defined.

Methods: We conducted a single-center, retrospective observational study at Tokyo Metropolitan Children's Medical Center. Patients with blood cultures yielding Moraxella spp. between April 2010 and March 2024 were identified. Clinical and microbiological data were extracted from electronic medical records. The primary outcome was all-cause 28-day mortality.

Results: Among 24 patients (28 episodes) with blood cultures positive for M. catarrhalis or M. osloensis, 13 episodes were excluded as contamination. Eleven patients (15 episodes) with confirmed bacteremia were analyzed: 8 episodes were due to M. osloensis and 7 to M. catarrhalis. Two patients had recurrent episodes (one patient with congenital nephrotic syndrome had four episodes of M. catarrhalis bacteremia). Overall, 10 of 11 patients (91%) had underlying immunodeficiency. Central venous catheters were present in 6 of 8 M. osloensis episodes, which were classified as catheter-associated bacteremia. In the M. catarrhalis group, pneumonia was the most likely focus in 2 of 7 episodes. Antimicrobial therapy commonly included cefepime or ampicillin-sulbactam for M. osloensis and cefotaxime or ampicillin-sulbactam for M. catarrhalis. The median duration of antimicrobial therapy was 14 days in both groups. No patients required pediatric intensive care unit (PICU) care and no 28-day mortality occurred.

Conclusion: The patients with bacteremia due to M. osloensis or M. catarrhalis had favorable clinical outcomes.

背景:莫拉菌属是上呼吸道的共生生物,仅偶尔引起侵袭性感染。小儿莫拉菌血症极为罕见,其临床特征和最佳管理仍然不明确。方法:我们在东京都儿童医学中心进行了一项单中心、回顾性观察研究。在2010年4月至2024年3月期间确定了血液培养产生莫拉菌的患者。临床和微生物学数据从电子病历中提取。主要终点为全因28天死亡率。结果:在24例(28次)卡他性支原体或奥斯洛支原体血培养阳性的患者中,13次被排除为污染。我们分析了11例确诊菌血症患者(15例),其中8例是由osloensis引起的,7例是由catarrhalis引起的。2例患者有反复发作(1例先天性肾病综合征患者有4次卡他氏分枝杆菌菌血症发作)。总体而言,11例患者中有10例(91%)存在潜在免疫缺陷。8例患者中有6例存在中心静脉导管。Osloensis发作,归类为导管相关性菌血症。在卡他性支原体组中,7次发作中有2次以肺炎为最可能的焦点。抗菌治疗通常包括头孢吡肟或氨苄西林-舒巴坦治疗奥斯洛支原体,头孢噻肟或氨苄西林-舒巴坦治疗卡塔卡支原体。两组患者抗菌药物治疗的中位持续时间均为14天。没有患者需要儿科重症监护病房(PICU)护理,没有28天死亡率发生。结论:奥斯洛支原体或卡他性支原体所致菌血症患者临床预后良好。
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引用次数: 0
Age-related variations in prostaglandin E-major urinary metabolite values in Japanese children. 日本儿童前列腺素e-主要尿代谢物值的年龄相关性变化。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70355
Takatoshi Maeyama, Ayaka Takashiba, Ayaha Hata, Keinosuke Hizuka, Ryutaro Saura, Yuri Etani, Shin-Ichiro Hagiwara

Background: Prostaglandin E-major urinary metabolite (PGE-MUM) is an emerging noninvasive biomarker used to evaluate clinical and endoscopic activity in patients with inflammatory bowel disease. Previous studies have shown that PGE-MUM values correlate with colonic inflammation in pediatric ulcerative colitis; however, reference values for children without inflammation have not been defined yet. This study aimed to determine the normal reference values of PGE-MUM in healthy pediatric subjects without inflammatory conditions.

Methods: Between December 2018 and January 2022, we prospectively enrolled 221 participants (cross-sectional study, aged 0-15 years) who were undergoing growth hormone stimulation testing for diagnostic purposes and exhibited no symptoms of inflammation or elevated C-reactive protein levels at a single pediatric center in Japan. PGE-MUM values were measured using a chemiluminescent enzyme immunoassay and assessed for age- and sex-related differences.

Results: A total of 218 participants were included in the analysis. Based on age, the participants were classified as young children (2-6 years, n = 147) and older children (7-14 years, n = 66); the reference ranges for the two groups were identified as 18.4-58.7 μg/g·Cr and 12.4-50.3 μg/g·Cr, respectively. The PGE-MUM values tended to decrease with increasing age; however, no significant sex-related differences were observed (median 29.1 μg/g·Cr for boys versus 30.4 μg/g·Cr for girls).

Conclusions: Healthy pediatric subjects generally have higher PGE-MUM values than healthy adults, with particularly elevated values in young children (2-6 years). These observations suggest that age-related variability must be taken into account when using PGE-MUM as a biomarker in young children with ulcerative colitis.

背景:前列腺素e -主要尿代谢物(PGE-MUM)是一种新兴的无创生物标志物,用于评估炎症性肠病患者的临床和内镜活性。先前的研究表明,PGE-MUM值与儿童溃疡性结肠炎的结肠炎症相关;然而,没有炎症的儿童的参考值尚未确定。本研究旨在确定无炎症的健康儿童PGE-MUM的正常参考值。方法:在2018年12月至2022年1月期间,我们前瞻性地招募了221名参与者(横断面研究,年龄0-15岁),他们在日本的一个儿科中心接受了生长激素刺激测试,用于诊断目的,并且没有出现炎症症状或c反应蛋白水平升高。使用化学发光酶免疫分析法测量PGE-MUM值,并评估年龄和性别相关差异。结果:共有218名参与者被纳入分析。根据年龄,参与者分为幼儿(2-6岁,n = 147)和较大的儿童(7-14岁,n = 66);两组的参考范围分别为18.4 ~ 58.7 μg/g·Cr和12.4 ~ 50.3 μg/g·Cr。PGE-MUM值随年龄的增加呈下降趋势;然而,没有观察到显著的性别相关差异(男孩的中位数为29.1 μg/g·Cr,女孩为30.4 μg/g·Cr)。结论:健康儿童受试者的PGE-MUM值通常高于健康成人,特别是幼儿(2-6岁)的PGE-MUM值升高。这些观察结果表明,在使用PGE-MUM作为溃疡性结肠炎幼儿的生物标志物时,必须考虑年龄相关的变异性。
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引用次数: 0
Assessment of pre-extubation pulmonary function in preterm infants with subsequent tracheomalacia. 气管软化症早产儿拔管前肺功能的评估。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70358
Masanori Wasa, Hisaya Hasegawa, Yosuke Yamada, Masae Mizogami, Kenta Ikeda, Shoichiro Kanabuchi

Background: Tracheomalacia, a common condition in neonates and children, is often associated with prematurity and prolonged mechanical ventilation. We aimed to investigate the characteristics of pulmonary function tests (PFTs) performed before extubation in preterm infants with subsequent diagnosis of tracheomalacia.

Methods: We retrospectively analyzed 44 preterm infants who underwent pre-extubation PFTs and subsequent flexible fiberoptic bronchoscopy (FFB) between April 2011 and May 2023. Nineteen infants were diagnosed with tracheomalacia by FFB (tracheomalacia group) and compared with 25 infants without the diagnosis (nontracheomalacia group). Tracheomalacia was defined as tracheal collapse during expiration with an increased ratio of the posterior membranous wall to tracheal cartilage observed during FFB. We compared patient characteristics and pulmonary function parameters, including static respiratory compliance (Crs) and crying vital capacity (CVC), between the two groups.

Results: The tracheomalacia group had significantly lower gestational age and birth weight. Pre-extubation Crs was significantly higher in the tracheomalacia group (1.23 vs. 0.97 mL/cmH2O/kg, p < 0.05), while CVC and maximal inspiratory pressure did not differ significantly.

Conclusion: Preterm infants who were subsequently diagnosed with tracheomalacia exhibited higher Crs before extubation. Pre-extubation Crs may be a potential predictor for the subsequent development of tracheomalacia in this population.

背景:气管软化症是新生儿和儿童的常见病,常与早产和机械通气时间延长有关。我们的目的是研究诊断为气管软化症的早产儿拔管前肺功能检查(PFTs)的特点。方法:我们回顾性分析了2011年4月至2023年5月期间接受拔管前PFTs和随后的柔性纤维支气管镜检查(FFB)的44名早产儿。19例患儿经FFB诊断为气管软化(气管软化组),25例患儿未诊断为气管软化(非气管软化组)。气管软化被定义为呼气时气管塌陷,在FFB期间观察到后膜壁与气管软骨的比例增加。我们比较了两组患者的特征和肺功能参数,包括静态呼吸顺应性(Crs)和呼出肺活量(CVC)。结果:气管软化组胎龄和出生体重明显降低。气管软化组拔管前Crs显著高于气管软化组(1.23 vs. 0.97 mL/cmH2O/kg, p)。结论:随后诊断为气管软化的早产儿拔管前Crs较高。拔管前Crs可能是该人群随后气管软化症发展的潜在预测因子。
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引用次数: 0
Nationwide daily exclusive breastfeeding rates during birth hospitalization in Japan. 日本出生住院期间全国每日纯母乳喂养率。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70374
Tsuyoshi Isojima, Noriko Kato, Naho Morisaki, Shiro Sugiura, Akinori Moriichi, Tetsuji Yokoyama

Objective: Breast milk provides optimal nutrition for infants, with exclusive breastfeeding recommended for the first 6 months of life. Education during birth hospitalization is vital to promote exclusive breastfeeding. However, standardized references for neonatal weight loss rates based on data including national representative, multi-hospital, with different policies remain unavailable.

Methods: Data of 4774 participants from 146 hospitals throughout Japan were obtained from the national growth survey. Among them, 3302 were eligible for the analysis. Daily exclusive breastfeeding rates and their associations with facility and individual characteristics during birth hospitalization were then analyzed. Additionally, weight loss percentile reference curves by delivery mode were established using the Lambda-Mu-Sigma method.

Results: The rate was 60.8% on day 0 and plateaued at approximately 25% on day 3. Being born in a facility with high exclusive breastfeeding rate (odds ratio [OR]: 333.3, 95% confidence interval [CI]: 15.2-7142, p < 0.0001), multiparity (OR: 1.9, 95% CI: 1.4-2.5, p < 0.0001), vaginal delivery (OR: 2.2, 95% CI: 1.6-3.1, p < 0.0001), and lower weight loss rate on day 3 (OR: 1.2, 95% CI: 1.1-1.2, p < 0.0001) were identified as independent factors, and facility-level differences accounted for 58.4% of the total variability for exclusive breastfeeding at discharge. Using the data from 831 neonates with exclusive breastfeeding at discharge, weight loss rate references were established.

Conclusion: This study revealed Japan's nationwide daily exclusive breastfeeding rates during birth hospitalization and their significant influencing factors. The established references may be useful in promoting exclusive breastfeeding clinically.

目的:母乳为婴儿提供最佳营养,建议在生命的前6个月纯母乳喂养。出生住院期间的教育对于促进纯母乳喂养至关重要。然而,基于国家代表性、多医院、不同政策的数据,新生儿体重减轻率的标准化参考资料仍然缺乏。方法:从全国增长调查中获得来自日本146家医院的4774名参与者的数据。其中有3302人符合分析条件。然后分析了出生住院期间每日纯母乳喂养率及其与设施和个人特征的关系。采用Lambda-Mu-Sigma方法建立了不同递送方式的减肥百分位数参考曲线。结果:第0天为60.8%,第3天稳定在25%左右。出生在纯母乳喂养率高的机构(优势比[OR]: 333.3, 95%可信区间[CI]: 15.2-7142, p)结论:本研究揭示了日本全国出生住院期间每日纯母乳喂养率及其显著影响因素。这些已建立的参考文献可能有助于临床推广纯母乳喂养。
{"title":"Nationwide daily exclusive breastfeeding rates during birth hospitalization in Japan.","authors":"Tsuyoshi Isojima, Noriko Kato, Naho Morisaki, Shiro Sugiura, Akinori Moriichi, Tetsuji Yokoyama","doi":"10.1111/ped.70374","DOIUrl":"https://doi.org/10.1111/ped.70374","url":null,"abstract":"<p><strong>Objective: </strong>Breast milk provides optimal nutrition for infants, with exclusive breastfeeding recommended for the first 6 months of life. Education during birth hospitalization is vital to promote exclusive breastfeeding. However, standardized references for neonatal weight loss rates based on data including national representative, multi-hospital, with different policies remain unavailable.</p><p><strong>Methods: </strong>Data of 4774 participants from 146 hospitals throughout Japan were obtained from the national growth survey. Among them, 3302 were eligible for the analysis. Daily exclusive breastfeeding rates and their associations with facility and individual characteristics during birth hospitalization were then analyzed. Additionally, weight loss percentile reference curves by delivery mode were established using the Lambda-Mu-Sigma method.</p><p><strong>Results: </strong>The rate was 60.8% on day 0 and plateaued at approximately 25% on day 3. Being born in a facility with high exclusive breastfeeding rate (odds ratio [OR]: 333.3, 95% confidence interval [CI]: 15.2-7142, p < 0.0001), multiparity (OR: 1.9, 95% CI: 1.4-2.5, p < 0.0001), vaginal delivery (OR: 2.2, 95% CI: 1.6-3.1, p < 0.0001), and lower weight loss rate on day 3 (OR: 1.2, 95% CI: 1.1-1.2, p < 0.0001) were identified as independent factors, and facility-level differences accounted for 58.4% of the total variability for exclusive breastfeeding at discharge. Using the data from 831 neonates with exclusive breastfeeding at discharge, weight loss rate references were established.</p><p><strong>Conclusion: </strong>This study revealed Japan's nationwide daily exclusive breastfeeding rates during birth hospitalization and their significant influencing factors. The established references may be useful in promoting exclusive breastfeeding clinically.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"68 1","pages":"e70374"},"PeriodicalIF":0.9,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147468815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antimicrobial stewardship program at a long-term care facility for severely disabled children and adults. 在严重残疾儿童和成人长期护理机构的抗菌药物管理计划。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70314
Nobuhiro Kanie, Yasuko Kudo, Koji Fukano, Masako Igari, Wakana Furushima, Ayaka Koide, Tetsuji Kaneko, Yoshihiko Morikawa, Sho Ishii, Kazuhiro Uda, Kahoru Fukuoka-Araki, Yuho Horikoshi

Background: The implementation of the antimicrobial stewardship program (ASP) has become a standard practice aimed at optimizing antimicrobial prescriptions to prevent further development of drug-resistant organisms. However, the challenges faced in long-term care facilities (LTCFs) often present a scarcity of human resources crucial for the effective implementation of ASP initiatives. Our study was to evaluate the impact of feasible ASP in the LTCF.

Methods: A quasi-experimental study was conducted at the Tokyo Metropolitan Fuchu Medical Center for the Disabled serving as a LTCF in Japan. The pre- and post-intervention periods were from April 2013 to March 2018 and from April 2018 to March 2019, respectively. Multidisciplinary ASP, including periodic educational interventions, utilization of internal treatment manuals, pre-authorization, and prospective audit and feedback on prescribing practices, was administered by the infectious diseases team from the Tokyo Metropolitan Children's Medical Center. We compared days of antimicrobial therapy (DOT) and resistance rates between the pre- and post-intervention phases.

Results: Following the intervention, there was a noteworthy decline in the use of oral broad-spectrum antimicrobials (macrolides, fluoroquinolones, and third-generation cephalosporins), demonstrating a statistically significant level of change (p < 0.05). Throughout the study period, resistance rates of Escherichia coli and Pseudomonas aeruginosa to meropenem and levofloxacin remained largely stable, with no notable changes observed.

Conclusion: In this study, extending the expertise of antimicrobial stewardship from an adjoining acute-care facility to a LTCF was associated with a 42% reduction in the use of oral broad-spectrum antimicrobials.

背景:抗菌药物管理计划(ASP)的实施已成为一种标准做法,旨在优化抗菌药物处方,以防止耐药菌的进一步发展。然而,长期护理机构(ltcf)面临的挑战往往是缺乏人力资源,这对有效实施ASP计划至关重要。我们的研究是评估可行的ASP对LTCF的影响。方法:在日本东京大都会府中残疾人医疗中心作为LTCF进行准实验研究。干预前后分别为2013年4月至2018年3月和2018年4月至2019年3月。多学科ASP由东京都儿童医疗中心传染病小组实施,包括定期教育干预、使用内部治疗手册、预授权以及对处方做法的前瞻性审计和反馈。我们比较了干预前后的抗菌药物治疗天数和耐药率。结果:干预后,口服广谱抗菌药物(大环内酯类药物、氟喹诺酮类药物和第三代头孢菌素)的使用显著下降,显示出统计学上显著的变化水平(p结论:在本研究中,将抗菌药物管理专业知识从毗邻的急症护理设施扩展到LTCF,与口服广谱抗菌药物的使用减少42%相关。
{"title":"Antimicrobial stewardship program at a long-term care facility for severely disabled children and adults.","authors":"Nobuhiro Kanie, Yasuko Kudo, Koji Fukano, Masako Igari, Wakana Furushima, Ayaka Koide, Tetsuji Kaneko, Yoshihiko Morikawa, Sho Ishii, Kazuhiro Uda, Kahoru Fukuoka-Araki, Yuho Horikoshi","doi":"10.1111/ped.70314","DOIUrl":"https://doi.org/10.1111/ped.70314","url":null,"abstract":"<p><strong>Background: </strong>The implementation of the antimicrobial stewardship program (ASP) has become a standard practice aimed at optimizing antimicrobial prescriptions to prevent further development of drug-resistant organisms. However, the challenges faced in long-term care facilities (LTCFs) often present a scarcity of human resources crucial for the effective implementation of ASP initiatives. Our study was to evaluate the impact of feasible ASP in the LTCF.</p><p><strong>Methods: </strong>A quasi-experimental study was conducted at the Tokyo Metropolitan Fuchu Medical Center for the Disabled serving as a LTCF in Japan. The pre- and post-intervention periods were from April 2013 to March 2018 and from April 2018 to March 2019, respectively. Multidisciplinary ASP, including periodic educational interventions, utilization of internal treatment manuals, pre-authorization, and prospective audit and feedback on prescribing practices, was administered by the infectious diseases team from the Tokyo Metropolitan Children's Medical Center. We compared days of antimicrobial therapy (DOT) and resistance rates between the pre- and post-intervention phases.</p><p><strong>Results: </strong>Following the intervention, there was a noteworthy decline in the use of oral broad-spectrum antimicrobials (macrolides, fluoroquinolones, and third-generation cephalosporins), demonstrating a statistically significant level of change (p < 0.05). Throughout the study period, resistance rates of Escherichia coli and Pseudomonas aeruginosa to meropenem and levofloxacin remained largely stable, with no notable changes observed.</p><p><strong>Conclusion: </strong>In this study, extending the expertise of antimicrobial stewardship from an adjoining acute-care facility to a LTCF was associated with a 42% reduction in the use of oral broad-spectrum antimicrobials.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"68 1","pages":"e70314"},"PeriodicalIF":0.9,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146125722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Transcutaneous and precordial Doppler ultrasonography to confirm correct intraosseous access placement: A case report. 经皮及心前多普勒超声确认正确的骨内通道放置:1例报告。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70336
Tomoki Ueda, Yuji Hirayama, Tomoya Ito, Taiki Kojima
{"title":"Transcutaneous and precordial Doppler ultrasonography to confirm correct intraosseous access placement: A case report.","authors":"Tomoki Ueda, Yuji Hirayama, Tomoya Ito, Taiki Kojima","doi":"10.1111/ped.70336","DOIUrl":"10.1111/ped.70336","url":null,"abstract":"","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"68 1","pages":"e70336"},"PeriodicalIF":0.9,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12915516/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146150247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Analysis of intracellular organelles in neurons differentiated from iPSCs of Chédiak-Higashi syndrome patients. ch<s:1> - higashi综合征患者iPSCs分化神经元胞内细胞器的分析。
IF 0.9 4区 医学 Q3 PEDIATRICS Pub Date : 2026-01-01 DOI: 10.1111/ped.70326
Yasuko Nakamura, Masataka Ito, Akira Senoo, Shigeharu Oh, Akira Niwa, Yu Hashimoto, Hidetoshi Hagiwara, Yasuhiro Uemura, Tadataka Tsuji, Hidenori Ohnishi, Yasuo Horikoshi, Akira Shiraishi, Yumi Oshiro, Masataka Ishimura, Ryo Hanaki, Masahiro Hirayama, Kohsuke Imai, Osamu Ohara, Megumu K Saito, Ikuroh Ohsawa, Shigeaki Nonoyama

Background: Clinical manifestations of Chédiak-Higashi syndrome (CHS), an autosomal recessive disorder caused by LYST gene variants, include immunodeficiency and neurologic deficits. This study investigated the effects of defective LYST on neurodegenerative features by the morphological analysis of organelles in dopaminergic neurons differentiated from induced pluripotent stem cells (iPSCs) derived from CHS patients.

Methods: iPSCs derived from CHS patients were analyzed by immunostaining with antibodies against microtubule-associated protein 2 and tyrosine hydroxylase, periodic acid-Schiff (PAS) staining, electron microscopy, and staining with a fluorescent probe to monitor autophagy.

Results: iPSC-derived neurons contained PAS-positive giant granules and lipofuscin-like granules. Electron microscopy revealed enlarged lysosomes with electron-dense granules and filament-like structures. The number and brightness of autophagosomes and autolysosomes were markedly increased in CHS iPSC-derived neurons under basal culture conditions. CHS iPSC-derived neuronal mitochondria were enlarged, polymorphic, and hypertrophic.

Conclusions: CHS iPSC-derived neurons contained abnormal organelles, including lysosomes, autophagosomes, and mitochondria, which may be related to the neurodegenerative features of CHS.

背景:chamesdiak - higashi综合征(CHS)是一种由LYST基因变异引起的常染色体隐性遗传病,临床表现包括免疫缺陷和神经功能障碍。本研究通过对来自CHS患者的诱导多能干细胞(iPSCs)分化的多巴胺能神经元细胞器的形态学分析,探讨LYST缺陷对神经退行性特征的影响。方法:采用抗微管相关蛋白2和酪氨酸羟化酶抗体免疫染色、周期性酸-希夫(PAS)染色、电镜和荧光探针染色检测自噬情况,对CHS患者的iPSCs进行分析。结果:ipsc来源的神经元含有pas阳性巨颗粒和脂褐素样颗粒。电镜显示溶酶体增大,有电子致密颗粒和丝状结构。在基础培养条件下,CHS ipsc来源的神经元自噬体和自溶体的数量和亮度明显增加。CHS ipsc衍生的神经元线粒体扩大,多态和肥厚。结论:CHS ipsc源性神经元存在溶酶体、自噬体、线粒体等细胞器异常,可能与CHS神经退行性特征有关。
{"title":"Analysis of intracellular organelles in neurons differentiated from iPSCs of Chédiak-Higashi syndrome patients.","authors":"Yasuko Nakamura, Masataka Ito, Akira Senoo, Shigeharu Oh, Akira Niwa, Yu Hashimoto, Hidetoshi Hagiwara, Yasuhiro Uemura, Tadataka Tsuji, Hidenori Ohnishi, Yasuo Horikoshi, Akira Shiraishi, Yumi Oshiro, Masataka Ishimura, Ryo Hanaki, Masahiro Hirayama, Kohsuke Imai, Osamu Ohara, Megumu K Saito, Ikuroh Ohsawa, Shigeaki Nonoyama","doi":"10.1111/ped.70326","DOIUrl":"https://doi.org/10.1111/ped.70326","url":null,"abstract":"<p><strong>Background: </strong>Clinical manifestations of Chédiak-Higashi syndrome (CHS), an autosomal recessive disorder caused by LYST gene variants, include immunodeficiency and neurologic deficits. This study investigated the effects of defective LYST on neurodegenerative features by the morphological analysis of organelles in dopaminergic neurons differentiated from induced pluripotent stem cells (iPSCs) derived from CHS patients.</p><p><strong>Methods: </strong>iPSCs derived from CHS patients were analyzed by immunostaining with antibodies against microtubule-associated protein 2 and tyrosine hydroxylase, periodic acid-Schiff (PAS) staining, electron microscopy, and staining with a fluorescent probe to monitor autophagy.</p><p><strong>Results: </strong>iPSC-derived neurons contained PAS-positive giant granules and lipofuscin-like granules. Electron microscopy revealed enlarged lysosomes with electron-dense granules and filament-like structures. The number and brightness of autophagosomes and autolysosomes were markedly increased in CHS iPSC-derived neurons under basal culture conditions. CHS iPSC-derived neuronal mitochondria were enlarged, polymorphic, and hypertrophic.</p><p><strong>Conclusions: </strong>CHS iPSC-derived neurons contained abnormal organelles, including lysosomes, autophagosomes, and mitochondria, which may be related to the neurodegenerative features of CHS.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"68 1","pages":"e70326"},"PeriodicalIF":0.9,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146213750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Pediatrics International
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